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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in association with an adenocarcinoma: a case report.

Warth A, Herpel E, Schmähl A, Storz K, Schnabel PA - J Med Case Rep (2008)

Bottom Line: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder and information on this disease is limited, especially with regard to its management and prognosis.Here we report on a 60-year-old female patient with DIPNECH and an associated pulmonary adenocarcinoma.This case contributes to a better understanding of the disorder and its associated pathologies.

View Article: PubMed Central - HTML - PubMed

Affiliation: Institute of Pathology, University Hospital Heidelberg, Heidelberg, Germany. arne.warth@med.uni-heidelberg.de

ABSTRACT

Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder and information on this disease is limited, especially with regard to its management and prognosis. It has become generally accepted that DIPNECH is a precursor lesion to pulmonary carcinoid tumors.

Case presentation: Here we report on a 60-year-old female patient with DIPNECH and an associated pulmonary adenocarcinoma.

Conclusion: This case contributes to a better understanding of the disorder and its associated pathologies.

No MeSH data available.


Related in: MedlinePlus

Preoperative CT scans. The preoperative CT scans clearly demonstrate the main tumor in the upper lobe of the right lung.
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Figure 1: Preoperative CT scans. The preoperative CT scans clearly demonstrate the main tumor in the upper lobe of the right lung.

Mentions: Here we report on a patient with DIPNECH who coincidently developed a pulmonary adenocarcinoma. The 60-year-old female patient was initially referred to our hospital because computed tomography scans revealed a tumor-like lesion measuring 2.9 cm in its widest diameter in segment 2 (right upper lobe, posterior segment) of the right lung. Additionally, several lesions as large as 0.6 cm were evident in segments 4 (right middle lobe, lateral segment) and 6 (right lower lobe, superior segment) of the right lung. These lesions were suggested to represent metastases of the lesion in segment 2. A CT scan of the chest (Fig. 1) was indicated following detection of a pulmonary nodule in the right upper field on routine chest -x-ray. The patient had a 10 pack-year smoking history and complained of shortness of breath upon admission; the remaining review of symptoms was negative. Pre-operative diagnostics revealed arterial hypertension and moderate left ventricular hypertrophy and pulmonary function tests were unsuspicious (VC 143%, FEV1 128%). Since the CT findings raised the suspicion of a malignancy, a diagnostic thoracotomy with a concurrent sleeve lobectomy of the right upper lobe was performed in combination with a systematic lymphadenectomy. Pathological processing of the specimens revealed a 3.5 × 3 × 2.8 cm adenocarcinoma of a mixed subtype with partial neuroendocrine differentiation (Fig. 2). The tumor was strongly positive for CK7, CK18, TTF1 and SPA and focally positive for CEA, NSE and chromogranin A. The proliferation rate (Ki67) was 20–30%. Besides this main tumor there were multiple small metastases with a similar degree of differentiation in the upper right lobe as well as in segment 4 of the middle right lobe. Tumor infiltration of intrapulmonary and mediastinal lymph nodes was also present. Therefore, the TNM classification for the pulmonary adenocarcinoma was pT4, pN2 (16/27), pM1, G3. However, further processing of the multiple small lesions in the upper and middle lobe revealed five foci less than 5 mm in diameter with a different trabecular and nest-like morphology (Fig. 2). In these lesions, the cells were strongly positive for CD56, synaptophysin, NSE and chromogranin A and focally positive for CK7, CK18, TTF1 with a proliferation rate (Ki67) of 1–2%. Therefore, the diagnosis of multiple tumorlets (microcarcinoids) was made. Due to the multicentricity of the lesions and a size of <5 mm in diameter, the correct diagnosis was DIPNECH. Lesions >5 mm are classified as carcinoids according to the current WHO classification[2]. There were no clinical symptoms suggestive of any proteins and/or hormones released, but interestingly, NSE (20 ng/ml) and CEA (33 ng/ml) were slightly elevated, whereas Cyfra was within the normal range (1.4 ng/ml). The postoperative course of the patient was uneventful. Six months after the operation the patient is still alive and no tumor recurrence has been detected so far.


Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in association with an adenocarcinoma: a case report.

Warth A, Herpel E, Schmähl A, Storz K, Schnabel PA - J Med Case Rep (2008)

Preoperative CT scans. The preoperative CT scans clearly demonstrate the main tumor in the upper lobe of the right lung.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2254641&req=5

Figure 1: Preoperative CT scans. The preoperative CT scans clearly demonstrate the main tumor in the upper lobe of the right lung.
Mentions: Here we report on a patient with DIPNECH who coincidently developed a pulmonary adenocarcinoma. The 60-year-old female patient was initially referred to our hospital because computed tomography scans revealed a tumor-like lesion measuring 2.9 cm in its widest diameter in segment 2 (right upper lobe, posterior segment) of the right lung. Additionally, several lesions as large as 0.6 cm were evident in segments 4 (right middle lobe, lateral segment) and 6 (right lower lobe, superior segment) of the right lung. These lesions were suggested to represent metastases of the lesion in segment 2. A CT scan of the chest (Fig. 1) was indicated following detection of a pulmonary nodule in the right upper field on routine chest -x-ray. The patient had a 10 pack-year smoking history and complained of shortness of breath upon admission; the remaining review of symptoms was negative. Pre-operative diagnostics revealed arterial hypertension and moderate left ventricular hypertrophy and pulmonary function tests were unsuspicious (VC 143%, FEV1 128%). Since the CT findings raised the suspicion of a malignancy, a diagnostic thoracotomy with a concurrent sleeve lobectomy of the right upper lobe was performed in combination with a systematic lymphadenectomy. Pathological processing of the specimens revealed a 3.5 × 3 × 2.8 cm adenocarcinoma of a mixed subtype with partial neuroendocrine differentiation (Fig. 2). The tumor was strongly positive for CK7, CK18, TTF1 and SPA and focally positive for CEA, NSE and chromogranin A. The proliferation rate (Ki67) was 20–30%. Besides this main tumor there were multiple small metastases with a similar degree of differentiation in the upper right lobe as well as in segment 4 of the middle right lobe. Tumor infiltration of intrapulmonary and mediastinal lymph nodes was also present. Therefore, the TNM classification for the pulmonary adenocarcinoma was pT4, pN2 (16/27), pM1, G3. However, further processing of the multiple small lesions in the upper and middle lobe revealed five foci less than 5 mm in diameter with a different trabecular and nest-like morphology (Fig. 2). In these lesions, the cells were strongly positive for CD56, synaptophysin, NSE and chromogranin A and focally positive for CK7, CK18, TTF1 with a proliferation rate (Ki67) of 1–2%. Therefore, the diagnosis of multiple tumorlets (microcarcinoids) was made. Due to the multicentricity of the lesions and a size of <5 mm in diameter, the correct diagnosis was DIPNECH. Lesions >5 mm are classified as carcinoids according to the current WHO classification[2]. There were no clinical symptoms suggestive of any proteins and/or hormones released, but interestingly, NSE (20 ng/ml) and CEA (33 ng/ml) were slightly elevated, whereas Cyfra was within the normal range (1.4 ng/ml). The postoperative course of the patient was uneventful. Six months after the operation the patient is still alive and no tumor recurrence has been detected so far.

Bottom Line: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder and information on this disease is limited, especially with regard to its management and prognosis.Here we report on a 60-year-old female patient with DIPNECH and an associated pulmonary adenocarcinoma.This case contributes to a better understanding of the disorder and its associated pathologies.

View Article: PubMed Central - HTML - PubMed

Affiliation: Institute of Pathology, University Hospital Heidelberg, Heidelberg, Germany. arne.warth@med.uni-heidelberg.de

ABSTRACT

Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder and information on this disease is limited, especially with regard to its management and prognosis. It has become generally accepted that DIPNECH is a precursor lesion to pulmonary carcinoid tumors.

Case presentation: Here we report on a 60-year-old female patient with DIPNECH and an associated pulmonary adenocarcinoma.

Conclusion: This case contributes to a better understanding of the disorder and its associated pathologies.

No MeSH data available.


Related in: MedlinePlus